Roux-en-Y gastric bypass surgery in the management of familial partial lipodystrophy type 1
A Melvin, C Adams, C Flanagan, L Gaff… - The Journal of …, 2017 - academic.oup.com
A Melvin, C Adams, C Flanagan, L Gaff, B Gratton, F Gribble, G Roberts, RK Semple…
The Journal of Clinical Endocrinology & Metabolism, 2017•academic.oup.comContext Familial partial lipodystrophy type 1 (FPLD1) is an extreme form of central adiposity,
with peripheral lipodystrophy associated with severe manifestations of the metabolic
syndrome, often poorly responsive to standard therapeutic approaches. Body mass index in
FPLD1 varies but, in many cases, is below the level at which metabolic surgery is usually
considered as a therapeutic option. Design We detailed the metabolic response to gastric
bypass surgery of three patients with FPLD1, refractory to medical therapy. Results Roux-en …
with peripheral lipodystrophy associated with severe manifestations of the metabolic
syndrome, often poorly responsive to standard therapeutic approaches. Body mass index in
FPLD1 varies but, in many cases, is below the level at which metabolic surgery is usually
considered as a therapeutic option. Design We detailed the metabolic response to gastric
bypass surgery of three patients with FPLD1, refractory to medical therapy. Results Roux-en …
Context
Familial partial lipodystrophy type 1 (FPLD1) is an extreme form of central adiposity, with peripheral lipodystrophy associated with severe manifestations of the metabolic syndrome, often poorly responsive to standard therapeutic approaches. Body mass index in FPLD1 varies but, in many cases, is below the level at which metabolic surgery is usually considered as a therapeutic option.
Design
We detailed the metabolic response to gastric bypass surgery of three patients with FPLD1, refractory to medical therapy.
Results
Roux-en-Y gastric bypass (RYGB) was associated with weight loss and substantial improvements in glycemic control and insulin sensitivity. All three patients were able to stop using insulin. Glucose tolerance testing in one patient demonstrated an increase in L-cell–derived gut hormone responses postoperatively.
Conclusion
RYGB surgery substantially improved glycemic control in three patients with FPLD1, two of whom had body mass indices below 30 kg/m2. RYGB should be considered in patients with partial lipodystrophy and refractory metabolic disease.
Oxford University Press